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392,393 Iron accumulates in the parenchymal cells of various organs in HH patients, generating ROS through the Fenton reaction, resulting in ferroptosis and several serious chronic complications, including heart disease, diabetes, and cirrhosis

Relative to glutathione availability, one of the most important considerations has been to properly maintain the availability of cysteine in the blood as that is known to be the rate-limiting substrate for glutathione resynthesis[3]

without a sufficient supply of cysteine, the entire glutathione synthesis pathway grinds to a halt, leaving cells vulnerable to oxidative damage

James At Dr